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Vol. 101. Issue 10.
Pages 820-826 (December 2010)
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Vol. 101. Issue 10.
Pages 820-826 (December 2010)
Practical dermatology
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Clinical Guidelines for the Diagnosis and Treatment of Dermatitis Herpetiformis
Guía clínica de diagnóstico y tratamiento de la dermatitis herpetiforme
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J.E. Herrero-González
Departamento de Dermatología, Hospital del Mar, Parc de Salut Mar, Institut Municipal d’Investigació Mèdica, Parc de Recerca Biomèdica de Barcelona, Barcelona, Spain
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Abstract

Dermatitis herpetiformis is an autoimmune blistering disease that appears as a cutaneous manifestation of gluten intolerance. It is one of a group of disorders that have gluten sensitivity in common, including celiac disease and gluten ataxia. Patients with dermatitis herpetiformis present with a pruritic papulovesicular rash on extensor surfaces and on the buttocks. Immunological studies demonstrate the presence of specific immunoglobulin (Ig) A antiendomysial and antitransglutaminase antibodies. The finding of granular deposits of IgA along the dermal-epidermal junction is pathognomonic of dermatitis herpetiformis. Treatment of dermatitis herpetiformis is based on a life-long, strict gluten-free diet, which improves all clinical aspects of gluten sensitivity, and dapsone, a drug that is only effective for the skin manifestations.

Keywords:
Celiac disease
Dapsone
Dermatitis herpetiformis
Duhring disease
Gluten
Resumen

La dermatitis herpetiforme es una enfermedad ampollosa autoinmune que aparece como expresión cutánea de la intolerancia al gluten. Forma parte de un abanico de patologías que tienen en común la sensibilidad a este componente, entre las cuales se encuentra la celiaquía y la ataxia por gluten. Los pacientes con dermatitis herpetiforme presentan una erupción papulovesicular pruriginosa de predominio en superficies de extensión y nalgas. El estudio inmunológico demuestra la presencia de anticuerpos específicos IgA antiendomisio y antitransglutaminasa. El hallazgo de depósitos granulares de IgA en la unión dermoepidérmica es patognomónico de la dermatitis herpetiforme. El tratamiento se basa en dos pilares: mantener indefinidamente una dieta estricta libre de este componente (la cual mejora todas las formas clínicas de la sensibilidad al gluten) y la dapsona, fármaco que es eficaz solo para las manifestaciones cutáneas.

Palabras clave:
Celiaquía
Dapsona
Dermatitis herpetiforme
Enfermedad de Duhring
Gluten
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References
[1.]
J. Marks, S. Shuster, A. Watson.
Small-bowel changes in dermatitis herpetiformis.
Lancet, 2 (1966), pp. 1280-1282
[2.]
A. Spurkland, G. Ingvarsson, E.S. Falk, I. Knutsen, L.M. Sollid, E. Thorsby.
Dermatitis herpetiformis and celiac disease are both primarily associated with the HLA-DQ (alpha 1*0501, beta 1*02) or the HLA-DQ (alpha 1*03, beta 1*0302) heterodimers.
Tissue Antigens, 49 (1997), pp. 29-34
[3.]
J.A. Sachs, J. Awad, D. McCloskey, C. Navarrete, H. Festenstein, E. Elliot, et al.
Different HLA associated gene combinations contribute to susceptibility for coeliac disease and dermatitis herpetiformis.
Gut, 27 (1986), pp. 515-520
[4.]
R.P. Hall, M.E. Sanders, R.J. Duquesnoy, S.I. Katz, S. Shaw.
Alterations in HLA-DP and HLA-DQ antigen frequency in patients with dermatitis herpetiformis.
J Invest Dermatol, 93 (1989), pp. 501-505
[5.]
M.C. Mazzilli, P. Ferrante, P. Mariani, E. Martone, F. Petronzelli, P. Triglione, et al.
A study of Italian pediatric celiac disease patients confirms that the primary HLA association is to the DQ (alpha 1*0501, beta 1*0201) heterodimer.
Hum Immunol, 33 (1992), pp. 133-139
[6.]
M. Congia, F. Frau, R. Lampis, R. Frau, R. Mele, F. Cucca, et al.
A high frequency of the A30, B18, DR3, DRw52, DQw2 extended haplotype in Sardinian celiac disease patients: further evidence that disease susceptibility is conferred by DQ A1□0501, B1□0201.
Tissue Antigens, 39 (1992), pp. 78-83
[7.]
Z. Fronek, M.M. Cheung, A.M. Hanbury, M.F. Kagnoff.
Molecular analysis of HLA DP and DQ genes associated with dermatitis herpetiformis.
J Invest Dermatol, 97 (1991), pp. 799-802
[8.]
P. Price, C. Witt, R. Allcock, D. Sayer, M. Garlepp, C.C. Kok, et al.
The genetic basis for the association of the 8.1 ancestral haplotype (A1, B8, DR3) with multiple immunopathological diseases.
Immunol Rev, 167 (1999), pp. 257-274
[9.]
J. Alonso-Llamazares, L.E. Gibson, R.S. Rogers.
Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience.
Int J Dermatol, 46 (2007), pp. 910-919
[10.]
E. Marietta, K. Black, M. Camilleri, P. Krause, R.S. Rogers, C. David, et al.
A new model for dermatitis herpetiformis that uses HLA-DQ8 transgenic NOD mice.
J Clin Invest, 114 (2004), pp. 1090-1097
[11.]
M. Caproni, E. Antiga, L. Melani, P. Fabbri.
The Italian Group for Cutaneous Immunopathology. Guidelines for the diagnosis and treatment of dermatitis herpetiformis.
J Eur Acad Dermatol Venereol, 23 (2009), pp. 633-638
[12.]
T.L. Reunala.
Dermatitis herpetiformis.
Clin Dermatol, 19 (2001), pp. 728-736
[13.]
A.S. Oxentenko, J.A. Murray.
Celiac disease and dermatitis herpetiformis: the spectrum of gluten-sensitive enteropathy.
Int J Dermatol, 42 (2003), pp. 585-587
[14.]
R. Suárez-Fernández, A. España-Alonso, J. Herrero-González, J.M. Mascaró-Galy.
Practical management of the most common autoimmune bullous diseases.
Actas Dermosifiliogr, 99 (2008), pp. 441-455
[15.]
L. Fry, P. Keir, R.M. McMinn, J.D. Cowan, A.V. Hoffbrand.
Small-intestinal structure and function and haematological changes in dermatitis herpetiformis.
Lancet, 2 (1967), pp. 729-733
[16.]
S. Shuster, A.J. Watson, J. Marks.
Coeliac syndrome in dermatitis herpetiformis.
Lancet, 1 (1968), pp. 1101-1106
[17.]
J.B. Meer, V. der.
Granular deposits of immunoglobulins in the skin of patients with dermatitis herpetiformis. An immunofluorescent study.
Br J Dermatol, 81 (1969), pp. 493-503
[18.]
J.J. Zone, L. Meyer, M.J. Petersen.
Deposition of granular IgA relative to clinical lesions in dermatitis herpetiformis.
Arch Dermatol, 132 (1996), pp. 912-918
[19.]
W. Dieterich, T. Ehnis, M. Bauer, P. Donner, U. Volta, E.O. Riecken, et al.
Identification of tissue transglutaminase as the autoantigen of celiac disease.
Nat Med, 3 (1997), pp. 797-801
[20.]
W. Dieterich, E. Laag, L. Bruckner-Tuderman, T. Reunala, S. Kárpáti, T. Zágoni, et al.
Antibodies to tissue transglutaminase as serologic markers in patients with dermatitis herpetiformis.
J Invest Dermatol, 113 (1999), pp. 133-136
[21.]
M. Sardy, S. Karpati, B. Merkl, M. Paulsson, N. Smyth.
Epidermal transglutaminase (TGase 3) is the autoantigen of dermatitis herpetiformis.
J Exp Med, 195 (2002), pp. 747-757
[22.]
V. Kumar, H. Zane, N. Kaul.
Serologic markers of gluten-sensitive enteropathy in bullous diseases.
Arch Dermatol, 128 (1992), pp. 1474-1478
[23.]
V. Kumar, E. Hemedinger, T. Chorzelski, E.H. Beutner, J.E. Valeski, C. Kowalewski.
Reticulin and endomysial antibodies in bullous diseases. Comparison of specificity and sensitivity.
Arch Dermatol, 123 (1987), pp. 1179-1182
[24.]
T.P. Chorzelski, J. Sulej, H. Tchorzewska, S. Jablonska, E.H. Beutner, V. Kumar.
IgA class endomysium antibodies in dermatitis herpetiformis and coeliac disease.
Ann N Y Acad Sci, 420 (1983), pp. 325-334
[25.]
U. Volta, N. Molinaro, R. De Franchis, L. Forzenigo, M. Landoni, D. Fratangelo, et al.
Correlation between IgA antiendomysial antibodies and subtotal villous atrophy in dermatitis herpetiformis.
J Clin Gastroenterol, 14 (1992), pp. 298-301
[26.]
I.R. Korponay-Szabo, Z. Vecsei, R. Kiraly, I. Dahlbom, F. Chirdo, E. Nemes, et al.
Deamidated gliadin peptides form epitopes that transglutaminase antibodies recognize.
J Pediatr Gastroenterol Nutr, 46 (2008), pp. 253-261
[27.]
E.V. Marietta, S. Rashtak, J.A. Murray.
Correlation analysis of celiac sprue tissue transglutaminase and deamidated gliadin IgG/IgA.
World J Gastroenterol, 15 (2009), pp. 845-848
[28.]
D. Villalta, M.G. Alessio, M. Tampoia, E. Tonutti, I. Brusca, M. Bagnasco, et al.
Diagnostic accuracy of IgA anti-tissue transglutaminase antibody assays in celiac disease patients with selective IgA deficiency.
Ann N Y Acad Sci, 1109 (2007), pp. 212-220
[29.]
D. Villalta, M.G. Alessio, M. Tampoia, E. Tonutti, I. Brusca, M. Bagnasco, et al.
Testing for IgG class antibodies in celiac disease patients with selective IgA deficiency. A comparison of the diagnostic accuracy of 9 IgG anti-tissue transglutaminase, 1 IgG anti-gliadin and 1 IgG anti-deaminated gliadin peptide antibody assays.
Clin Chim Acta, 382 (2007), pp. 95-99
[30.]
C.M. Swinson, G. Slavin, E.C. Coles, C.C. Booth.
Coeliac disease and malignancy.
Lancet, 1 (1983), pp. 111-115
[31.]
H.M. Lewis, T. Renaula, J.J. Garioch, J.N. Leonard, J.S. Fry, P. Collin, et al.
Protective effect of gluten-free diet against development of lymphoma in dermatitis herpetiformis.
Br J Dermatol, 135 (1996), pp. 363-367
[32.]
B. Sigurgeirsson, B.A. Agnarsson, B. Lindelof.
Risk of lymphoma in patients with dermatitis herpetiformis.
Bmj, 308 (1994), pp. 13-15
[33.]
P. Collin, E. Pukkala, T. Reunala.
Malignancy and survival in dermatitis herpetiformis: a comparison with coeliac disease.
Gut, 38 (1996), pp. 528-530
[34.]
A.J. Swerdlow, S. Whittaker, L.M. Carpenter, J.S.C. English.
Mortality and cancer incidence in patients with dermatitis herpetiformis: a cohort study.
Br J Dermatol, 129 (1993), pp. 140-144
[35.]
N.R. Lewis, R.F.A. Logan, R.B. Hubbard, J. West.
No increase in risk of fracture, malignancy or mortality in dermatitis herpetiformis: a cohort study.
Aliment Pharmacol Ther, 27 (2008), pp. 1140-1147
[36.]
G.K. Holmes, P. Prior, M.R. Lane, D. Pope, R.N. Allan.
Malignancy in coeliac disease--effect of a gluten free diet.
Gut, 30 (1989), pp. 333-338
[37.]
C.M. Hardman, J. Garioch, J.N. Leonard, H.J.W. Thomas, M.M. Walker, J.E. Lortan, et al.
Absence of toxicity of oats in patients with dermatitis herpetiformis.
N Engl J Med, 337 (1997), pp. 1884-1887
[38.]
J.J. Garioch, H. Lewis, S.A. Sargent, J.N. Leonard, L. Fry.
25 years’ experience of a gluten-free diet in the treatment of dermatitis herpetiformis.
Br J Dermatol, 131 (1994), pp. 541-545
[39.]
M.T. Bardella, C. Fredella, C. Trovato, E. Ermacora, R. Cavalli, V. Saladino, et al.
Long-term remission in patients with dermatitis herpetiformis on a normal diet.
Br J Dermatol, 149 (2003), pp. 968-971
[40.]
S.H. Wakelin.
Handbook of systemic drug treatment in Dermatology.
Manson Publishing, (2002),
[41.]
M.D. Coleman.
Dapsone: modes of action, toxicity and possible strategies for increasing patient tolerance.
Br J Dermatol, 129 (1993), pp. 507-513
[42.]
R. Prussick, M.A. Ali, D. Rosenthal, G. Guyatt.
The protective effect of vitamin E on the hemolysis associated with dapsone treatment in patients with dermatitis herpetiformis.
Arch Dermatol, 128 (1992), pp. 210-213
[43.]
L.E. Rhodes, M. Tingle, B.K. Park, P. Chu, J.L. Verbov, P.S. Friedmann.
Cimetidine improves the therapeutic/toxic ratio of dapsone in patients on chronic dapsone therapy.
Br J Dermatol, 132 (1995), pp. 257-262
Copyright © 2010. Academia Española de Dermatología y Venereología and Elsevier España, S.L.
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